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CARDIAC MASSES Nick Tehrani, MD Outline General Primary Cardiac tumors Benign Malignant Secondary Cardiac Tumors Benign Malignant Q U I Z TIME General Neoplasia of the heart or pericardium is more likely to be: Secondary, Primary than General Prevalence: Primary tumors <3/10,000 autopsies Over ¾ are benign Secondary 20-40 x more common Present in upto 20% of patients dying of malignancy General Presentation: Embolization Systemic Pulmonary (less common) Obstruction Arrhythmia Tamponade Direct compression of coronary artery Intramyocardial Conduction disturbances Global dysfunction due to infiltration What is this? Potential Pitfalls Reverberation artifact Change depth and gain Hiatal hernia Fills the Rt. Or Lt. Atrium Drink Pepsi …Normal Anatomic Variants Crista terminalis Normal anatomic ridge Eustatian valve Attaches to the anterior lip of the IVC Post transplant suture lines All kinds of funny masses Intraoperative inversion of the Lt atrial appendage Anatomy Crista Eustatian V. What is this? Outline General Primary Cardiac tumors Benign Primary Cardiac Tumors Benign Myxoma Papillary fibroelastoma Fibroma Lipoma Rhabdomyomas Benign Primary Cardiac Tumors Myxomas: The most common primary cardiac tumor in the Age > 35 75% in the Lt. Atrium near fossa ovalis 15% Rt. Atrium 5-10% Lt. Ventricle Will discuss further 5% multiple sites Benign Primary Cardiac Tumors …Myxomas: Grossly Typically pedunculated Gelatinous consistency Friable Histologically Copious mucopolysaccharide stroma Scattered solitary or clustered polygonal cells. Left atrial Myxoma Benign Primary Cardiac Tumors …Myxomas: Extra cardiac manifestations suggestive of Collagen Vascular dz : Fever ESR elevation Anemia Thrombocythemia Circulating autoantibodies Benign Primary Cardiac Tumors …Myxomas: Etiology of immunologic manifestations ??? ? Tumor necrosis Anitimyocardial antibody titers decline post resection Benign Primary Cardiac Tumors Management of Myxomas: High propensity for embolization Surgical results excellent Decision to operate: Lt. versus Rt. Sided tumor Sx. Age Co morbidities Benign Primary Cardiac Tumors Familial pattern of Myxomas with autosomal dominant features Age < 30 Bad actor… Any guesses? Carney Complex Benign Primary Cardiac Tumors …Carney Complex: Multiple lentigines and blue nevi Peripheral myxoid tumors (cutaneous myxoma, myxoid mamary fibroadenoma) Psamommatous melanotic schwannoma Endocrine over activity (Cushing’s syndrome, pituitary adenomas, testicular Sertoli cell tumors) Multiple recurrent cardiac masses Benign Primary Cardiac Tumors …Carney Complex: Genes located on chromosomes 2p 17q2 Family members should be screened when an index case is identified Primary Cardiac Tumors Benign Myxoma Papillary fibroelastoma Benign Primary Cardiac Tumors Papillary Fibroelastoma: (aka Papilloma) The most common valvular tumor, followed by Sarcoma Melanoma Present in all age groups, but most commonly in Age>60 Benign Primary Cardiac Tumors …Papillary Fibroelastoma: Small tumors (<1cm) with homogeneous speckeled pattern Commonly pedunculated with multiple fronds Affect the Lt and Rt sides with same frequency Attach to Atrial surface of AV valves, and Ventricular surface of semilunar valves Benign Primary Cardiac Tumors …Papillary Fibroelastoma: Adults Aortic valve Children Tricuspid valve Rarely on endocardial surfaces Symptomatic only in the aortic position (other than embolic Sx) Ostial occlusion angina, sudden death Rarely valvular dysfunction Source of embolization in up to 30% of patients Benign Primary Cardiac Tumors Management of Papillary Fibroelastoma Surgical resection is not uniformly necessary Anticoagulation may be sufficient in many patients Surgery indicated for Embolic events Ostial occlusion Topol Textbook of Cardiovascular Medicine All fibroelastomas, “any size, any age” are to be resected Seward, ACC 2000 Primary Cardiac Tumors Benign Myxoma Papillary fibroelastoma Fibroma Benign Primary Cardiac Tumors Fibromas: Encapsulated, solitary tumors Frequently in the septal myocardium Often encroach on the conduction system as they grow Benign Primary Cardiac Tumors …Fibromas: With septal involvement V.Fib is often the first presentation Indications for surgical resection: Mechanical problems due to size Arrhythmogenic nidus Resection of septal fibromas is not always possible Benign Primary Cardiac Tumors Lipomas: Affect both myocardium and pericardium Can reach several centimeters in size Benign Primary Cardiac Tumors Rhabdomyoma: Most common tumor of the heart for Age < 30 yo Almost exclusively in children Associated with tuberous sclerosis Regression of tumor in infancy has been reported Outline General Primary Cardiac tumors Benign Malignant Malignant Primary Cardiac Tumors Angiosarcomas Rhabdomyosarcomas Mesotheliomas Lymphoma Intrapericardial Pheochromocytomas Malignant Primary Cardiac Tumors Angiosarcomas: Most common primary malignancy of the heart Malignant cells that form vascular channels Most commonly affect the Rt. Heart Rt. Atrium Pericardium Hemorrhagic effusion Thrombus Malignant Primary Cardiac Tumors …Angiosarcomas: Diffuse, irregularly shaped Mean survival one year Successful Rx with Chemo and XRT followed by transplant reported Malignant Primary Cardiac Tumors Rhabdomyosarcomas: Most commonly seen in adults No chamber selectivity No pericardial involvement Multiple sites of cardiac involvement is common Poor prognosis Limited success with resection and adjuvant Rx. Undifferentiated Sarcoma Malignant Primary Cardiac Tumors Mesotheliomas: Diffuse pericardial tumor Involve both parietal and visceral pericardium Superficially invade the myocardium Rarely invade the cardiac chambers Malignant Primary Cardiac Tumors …Mesotheliomas: Sx of pericarditis or tamponade Poor prognosis XTR or chemo only offer temporary improvement Malignant Primary Cardiac Tumors Lymphoma: Heart and pericardium are the only affected organs No predilection for any particular site Malignant Primary Cardiac Tumors Intrapericardial Pheochromocytomas: Soft, fleshy, highly vascular Anatomic location AV groove Atrium Interatrial septum Coronary, Pulmonary, Aorticopulmonary regions Generally very difficult to resect Malignant Primary Cardiac Tumors Generalizations: Pulmonary vein mass Malignancy Lateral wall of the LA Malignancy Atrial septum Myxoma Outline General Primary Cardiac tumors Benign Malignant Secondary Cardiac Tumors Malignant Malignant Secondary Cardiac Tumors Incidence of Solid Tumors involving the heart: Lung Breast Soft tissue sarcomas Renal carcinoma Melanoma Leukemia and Lymphomas also common culprits What is this? Malignant Secondary Cardiac Tumors IVC tumors in General Renal carcinoma 80% 5 year survival for surgical resection of tumors migrating up the IVC Hepatoma Ovarian CA Outline General Primary Cardiac tumors Benign Malignant Secondary Cardiac Tumors Malignant Benign Benign Secondary Cardiac Tumors Hints: IVC tumor Long stringy appearance Seen many years post hysterectomy LEIOMYOMATOSIS Benign Secondary Cardiac Tumors Leiomyomatosis: Akin to Fibroids Controlled by hormone suppression Can degenerate into very low grade sarcoma Histologically, similar structures are present in the venous channels of the uterus HIV and cardiac tumors Kaposi’s sarcoma can involve the myocardium or pericardium Non-Hodgkin's lymphoma Can present as primary cardiac lymphoma When involving the heart usually diffusely infiltrative Variable results with chemo and XRT Another Benign Secondary CardiacTumor How about this one? APICAL MASSES Differential Dx for Apical Masses Thrombus NL Wall Motion Leofler’s Endomyocardial fibrosis (seen in tropics) Outline General Primary Cardiac tumors Benign Malignant Secondary Cardiac Tumors Malignant Benign Surgical Options Surgical Options Primary Cardiac Tumors Rare entity, <3/10,000 autopsies Surgical options Benign majority resectable 6% rate of recurrence Malignant seldom resectable Surgery often intended for tissue diagnosis (benign vs. malignant) Surgical Options If surgical resection is contemplated, thorough metastatic evaluation is necessary: CT Head Chest Abd Bone scan if indicated Bone marrow Bx if indicated Surgical Options Total of 28 patients have undergone orthotopic heart transplantation for inoperable primary cardiac tumors: 7 had benign histology Mean survival of 46 months 21 had malignant histology Mean survival 12 months Surgical Options OF the 7 with benign histology Fibroma 5 patients Rhabdomyoma 1 Pheochromocytoma 1 Survival range: 8 to 105 months Survival mean: 46 months No patients had recurrence of tumor Two deaths due to rejection Surgical Options OF the 21 with malignant histology Sarcoma 15 Malignant fibrohistiocytoma 3 Lymphoma 3 14/21 died between 1 and 36 months (mean 12 months) 13/14 died of recurrent mets 1/14 cause of death unknown 7 others; follow up 6 – 66 months one had recurrence malignancy Surgical Options OHT is promising for patients with unresectable benign neoplasias Surgical Options Malignant primary cardiac neoplasms not routinely considered for OHT due to: Concern for tumor recurrence Immunosuppression stimulation of tumor growth Surgical Options Patients with Primary malignant neoplasias need to be assessed on a case by case basis due to: Limited experience Heterogeneous nature of the malignancies Yet to be defined, role of: Adjuvant chemo 10/21 had received chemo XRT Q U I Z TIME Any Guesses?? The answer Is Ruptured Papillary Muscle Abcess Inflamatory tissue Any Guesses?? The answer Is Non-specific Inflamatory Tissue What is this? PE in Transit across PFO What is this? Hiatal Hernia What is this? Mediastinal Lymph node Myxoma with recent thrombosis What do you really see here?